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BRIEF COMMUNICATION
Year : 2019  |  Volume : 7  |  Issue : 2  |  Page : 73-75

Adult presentation of Haab's striae


1 Consultant Ophthalmologist, Spark Eye Hospital, Hyderabad, Telangana, India
2 Department of Ophthalmology, INHS Kalyani, Visakhapatnam, Andhra Pradesh, India
3 Department of Ophthalmology, Armed Forces Medical College, Pune, Maharashtra, India

Date of Submission01-Oct-2018
Date of Acceptance21-Jan-2019
Date of Web Publication21-Aug-2019

Correspondence Address:
Harikrishnan Vannadil
Spark Eye Hospital, Malakpet, Hyderabad - 500 036, Telangana
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jcor.jcor_78_18

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  Abstract 


In adults, Haab's striae are often an accidental finding during routine examination. The presence of Haab's striae points to an underlying event involving the eye during the developmental stage. Even though in most cases, the event might have been resolved, an attempt should be made to ascertain the cause for such a finding. Here, we report a case of a young adult with Haab's striae in one eye and a posttraumatic phthisical fellow eye. In the absence of any concrete history, we have to consider the possibility of glaucoma as well as trauma. Necessary investigations and follow-up are warranted in such cases.

Keywords: Congenital glaucoma, cornea, Haab's striae, ocular trauma


How to cite this article:
Vannadil H, Singh AD, Bhatta S. Adult presentation of Haab's striae. J Clin Ophthalmol Res 2019;7:73-5

How to cite this URL:
Vannadil H, Singh AD, Bhatta S. Adult presentation of Haab's striae. J Clin Ophthalmol Res [serial online] 2019 [cited 2019 Nov 20];7:73-5. Available from: http://www.jcor.in/text.asp?2019/7/2/73/264897



The origin of Descemet's membrane tears in children has been studied in depth. Any form of external or internal pressure in a pliable cranium can lead to the stretch of the cornea.[1],[2] The Descemet's membrane does not yield well to this stretch and results in tears perpendicular to the direction of trauma. The edges of the tear separate with growth of the cornea and the intervening zone becomes devoid of the endothelium.[1],[2] Over a period of time, after the inciting event has subsided, the intervening zone is covered with free endothelial cells with a thinner than normal Descemet's membrane.[1] This is evident with the cornea being transparent, on a slit-lamp examination. This clinical sign is known as Haab's striae.[1],[3]

Haab's striae have been a pathognomic sign of primary congenital glaucoma where the increased intraocular pressure caused the stretch and subsequent formation of Descemet's membrane tears. However, there are other conditions in which similar findings are described.[3],[4] These Descemet's membrane tears are also found in birth trauma and posterior polymorphous corneal dystrophy.[5],[6] In birth trauma, which is commonly attributed to forceps delivery, there can be various patterns of injury.[5],[7]


  Case Report Top


A 22-year-old male unmarried patient presented to our center in the month of January 2018. His presenting complaints were absence of vision in his right eye. The patient gave history of blunt trauma to his face during his childhood, while he was accidentally embroiled in an incident of major domestic violence among his relatives. Although the patient was unable to give exact dates, this incident dates back to a time when he was less than 1-year-old. This allegedly resulted him to lose vision in his right eye completely with no such complaints in his left eye. There is no history of any birth trauma. He denied any history of glare or photophobia in his left eye.

On examination, his right eye is phthisical with no perception of light. There was extensive vascularization of the cornea with irregular surface [Figure 1].
Figure 1: (a) The phthisical right eye of the patient showing corneal vascularization. (b) The left eye of the patient showing the Descemet's membrane striae

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The left eye was found to have a vision of 6/9 (LogMAR 0.20, 20/30) with a refraction of +0.25 Diopter (D) sphere/−0.50 × D cylinder (cyl) at 80°. The patient corrected to 6/6 (LogMAR 0.0, 20/20) with a prescription of −0.50 D cyl at 90°.

On slit-lamp examination, the ocular adnexa was found to be within normal limits with no signs suggestive of trauma. On examining the cornea, two lines separated by uniform thickness of 0.9 mm with intervening transparent area were found coursing from the superior cornea to inferior cornea through the nasal side, avoiding the pupillary axis [Figure 1]. On optical section, the lines were identified in the Descemet's membrane with mild corneal thinning in the intervening area [Figure 2]. The corneal diameter of the left eye was found to be 12.4 mm (white to white as measured by IOLMaster 400). There was no widening of the limbus. Rest of the anterior-segment examination was within normal limits. Fundoscopy revealed a normal-appearing nonglaucomatous optic disc with normal fundus [Figure 3].
Figure 2: Optical section of the left cornea showing the location of the striae

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Figure 3: 90D image of the optic disc of the left eye showing normal optic disc morphology

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Intraocular pressure with noncontact office tonometer was found to be in a range of 12–15 mmHg at different occasions. Gonioscopy revealed normal open angles in all quadrants. Visual field analysis by Humphrey Field Analyzer II did not reveal any glaucomatous changes. Central corneal thickness, axial length, and anterior-segment optical coherence tomography (AS-OCT) were not measured due to various reasons.

The patient was counseled regarding the poor potential of vision in his right eye. He was also advised for follow-up at our center for his left eye. The patient was not given any form of pharmacological agents for his usage.


  Discussion Top


Haab's striae are a finding that is considered as a hallmark of congenital glaucoma and birth trauma.[2],[6],[7],[8] It is mostly encountered in a pediatric population. Encountering Haab's striae in an adult may suggest a past trauma or an episode of elevated intraocular pressure.

Various studies have shown the presence of similar Descemet's membrane tears in cases of congenital glaucoma. These cases have a large cornea with characteristic optic disc changes with an active disease.[2],[4],[8] Other signs of advanced glaucoma accompany these conditions by the second decade of life. They may also have signs of anterior-segment disorders. Further, a successfully treated congenital glaucoma with good control of intraocular pressure is possible with medical or surgical intervention. In the successfully treated cases, there also occurs a reversal of optic disc findings, more so in patients controlled before the age of 1 year.[9] However, there are case reports which suggest that congenital glaucoma with specific genetic mutation can have asymmetric presentation with onset in adulthood where Haab's striae may be the only finding on clinical examination.[4] This aspect stresses on the requirement of close follow-up in this case. There also exists a requirement of a more detailed workup including AS-OCT, axial length, and OCT–retinal nerve fiber layer.

The absence of any such history or clinical findings in this patient excludes congenital glaucoma as a most likely diagnosis. The positive history of trauma to the face with a phthisical right eye points to an episode of trauma to the left eye as well. It is a possibility that the left eye had an episode of trauma with or without elevation of intraocular pressure. Subsequent regression of the condition may have possibly reduced the intraocular pressure, thus reversing the optic disc signs.[9],[10] With the absence of any visual field defects and normal angles on gonioscopy, resolved hyphema or traumatic uveitis is a possibility.

Since the origin of the insult to the Descemet's membrane is uncertain, it would be prudent to follow-up these patients in order to detect any other undiagnosed abnormality.

The aim of this case report is to explore the possible etiologies in a case of Haab's striae presenting in adulthood with no other telltale signs.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Spierer O, Cavuoto KM, Suwannaraj S, Chang TC. Anterior segment optical coherence tomography imaging of Haab striae. J Pediatr Ophthalmol Strabismus 2015;52:e55-8.  Back to cited text no. 1
    
2.
Yeung HH. Haab's striae with congenital glaucoma. J Pediatr Ophthalmol Strabismus 2010;47:128.  Back to cited text no. 2
    
3.
Cibis GW, Tripathi RC. The differential diagnosis of Descemet's tears (Haab's striae) and posterior polymorphous dystrophy bands. A clinicopathologic study. Ophthalmology 1982;89:614-20.  Back to cited text no. 3
    
4.
Khan AO, Aldahmesh MA, Mohamed JY, Alkuraya FS. Corneal enlargement without optic disk cupping in children with recessive CYP1B1 mutations. J AAPOS 2013;17:643-5.  Back to cited text no. 4
    
5.
Angell LK, Robb RM, Berson FG. Visual prognosis in patients with ruptures in Descemet's membrane due to forceps injuries. Arch Ophthalmol 1981;99:2137-9.  Back to cited text no. 5
    
6.
Levecq L, Francart D, Van Hollebeke I. Ruptures in Descemet's membrane due to forceps injuries: Appearance clinically and on optical coherence tomography. J Fr Ophtalmol 2014;37:173-5.  Back to cited text no. 6
    
7.
Lambert SR, Drack AV, Hutchinson AK. Longitudinal changes in the refractive errors of children with tears in Descemet's membrane following forceps injuries. J AAPOS 2004;8:368-70.  Back to cited text no. 7
    
8.
Mastropasqua L, Carpineto P, Ciancaglini M, Nubile M, Doronzo E.In vivo confocal microscopy in primary congenital glaucoma with megalocornea. J Glaucoma 2002;11:83-9.  Back to cited text no. 8
    
9.
Shaffer RN, Hetherington J Jr. The glaucomatous disc in infants. A suggested hypothesis for disc cupping. Trans Am Acad Ophthalmol Otolaryngol 1969;73:923-35.  Back to cited text no. 9
    
10.
Quigley HA. Childhood glaucoma: Results with trabeculotomy and study of reversible cupping. Ophthalmology 1982;89:219-26.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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